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The Normal and Neoplastic Perineurium: A Review

Piña-Oviedo, Sergio MD* †; Ortiz-Hidalgo, Carlos MD* ‡

Advances in Anatomic Pathology: May 2008 - Volume 15 - Issue 3 - p 147-164
doi: 10.1097/PAP.0b013e31816f8519
Review Articles

Peripheral nerves consist of 3 layers with differing characteristics: the endoneurium, perineurium, and epineurium. The perineurium represents a continuum with the pia-arachnoid from the central nervous system and extends distally with the sheath of capsular cells of peripheral sensorial organs and propioceptive receptors. It is made of layers of flattened cells surrounded by a basement membrane and collagen fibers, forming concentrically laminated structures around single nerve fascicles. Functionally, the perineurium modulates external stretching forces (that could be potentially harmful for nerve fibers), and along with endoneurial vessels, forms the blood-nerve barrier. Multiple pathologic conditions associated with the perineurium have been described. Perineurial invasion is considered an important prognostic factor in several malignant neoplasms. Perineuriomas are true benign infrequent perineurial cell neoplasms that have been divided in 2 categories: those with intraneural localization and a more common extraneural (soft tissue) group, including sclerosing and reticular variants. Sporadic cases of malignant perineuromas have been reported. Interestingly, neurofibromas and malignant peripheral nerve sheath tumors may also display perineurial cell differentiation. The histologic appearance of perineuriomas may overlap with other soft tissue spindle cell neoplasms. Immunohistochemistry is imperative for the diagnosis, although in certain cases ultrastructural studies may be needed. Typical perineuriomas are positive for epithelial membrane antigen, glucose transporter-1-1, and claudin-1, and negative for S-100 protein and neurofilaments. Perineuriomas have mostly simple karyotypes, with one or few chromosomal rearrangements or numerical changes and it seems that specific cytogenetic aberrations may correlate with perineurioma subtype.

*Laboratory of Cell and Tissue Biology, Universidad Panamericana School of Medicine, Donatello 59, Colonia Insurgentes Mixcoac

Instituto de Hematopatologia, Tamaulipas, Colonia Cuajimalpa

Department of Pathology, The American British Cowdray Medical Center, Colonia Las Americas, Mexico City, Mexico

Reprints: Carlos Ortiz-Hidalgo, MD, Department of Pathology, The American British Cowdray Medical Center, Calle Sur 132, No. 116, Colonia Las Americas, C.P. 01120 Mexico City, Mexico (e-mail:

© 2008 Lippincott Williams & Wilkins, Inc.